Anti-Nicotinic Acetylchone Receptor alpha 1 Antibody, clone 7C8.2 clone 7C8.2, from mouse

Acetylcholine receptor subunit alpha (UniProt: P02708; AChR nicotinic alpha 1) is encoded by the CHRNA1 (also known as ACHRA, CHNRA) gene (Gene ID: 1134) in human. Nicotinic Acetylcholine Receptor is a member of the ligan-gated ion channel family. It is a multi-pass membrane protein on the post-synaptic cell membrane. Nicotinic Ach receptor is a pentamer of two alpha chains and one each of the beta, delta, and gamma (in immature muscle) or epsilon (in mature muscle) chains. Two isoforms of this receptor have been described that are produced by alternative splicing. Isoform 1 is only expressed in skeletal muscle and isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus. It contains an extracellular domain (aa 21-255), four helical domains (aa 256-280; 288-306; 322-341; and 454-472) and a cytoplasmic domain (aa 342-453). After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. Defects in CHRNA1 gene are linked to Myasthenic syndrome, which is characterized by muscle weakness affecting the axial and limb muscles, and the ocular muscles that lead to ptosis and opthalmoplegia. The alpha subunit is the main focus for antibody binding in myasthenia gravis.